Mouse monoclonal [D33] to DesminDesmins are class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. Defects in Desmin are the cause of desmin-related cardio-skeletal myopathy (CSM) also known as desmin-related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Desmin belongs to the intermediate filament family.
Immunogen
Full length native protein purified from human muscle.
Key Feature
Clonality
Monoclonal
Isotype
IgG1
Clone Number
D33
Host Species
Mouse
Tested Applications
IHC-PWB
For IHC-P: Use at a dilution of 1:25-1:50 (ABC method). We suggest an incubation perios of 30 min at RT. For WB: Use at a dilution of 1:50-1:100. Optimal dilutions/concentrations should be determined by the researcher.
IHC-P analysis of human leiomyoma using Desmin antibody [D33].
Application
Formalin fixed paraffin embedded human leiomyoma stained with Desmin antibody .
Positive Control
Leiomyoma
Application Notes
For IHC-P: Use at a dilution of 1:25-1:50 (ABC method). We suggest an incubation perios of 30 min at RT. For WB: Use at a dilution of 1:50-1:100. Optimal dilutions/concentrations should be determined by the researcher.
Additional Information
Form
Liquid
Storage Instructions
Keep as concentrated solution, aliquot and store at 4°C. Do not freeze.